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低丙种球蛋白血症是否是 Good 综合征的一个固有特征?

Is hypogammaglobulinemia a constant feature in Good's syndrome?

出版信息

Int J Immunopathol Pharmacol. 2010 Oct-Dec;23(4):1275-9. doi: 10.1177/039463201002300434.

DOI:10.1177/039463201002300434
PMID:21244779
Abstract

Thymomas are rare tumors, which can be associated to a variety of paraneoplastic syndromes, including a fatal hypogammaglobulinemia, namely Good?s Syndrome (GS). Although the combination of thymoma and hypogammaglobulinemia is regarded as sufficient for diagnosis of Good?s syndrome, some thymoma patients with a clear clinical picture of immunodeficiency present normal levels of immunoglobulins. We describe the case of a patient, with a 20-year history of thymoma, who underwent several operations and lines of chemotherapy, and suffered from recurrent infections, including one rare skin infection from Pseudoallescheria boydii. The patient constantly presented normal levels of gammaglobulins.

摘要

胸腺瘤是一种罕见的肿瘤,可与多种副肿瘤综合征相关,包括致命性低丙种球蛋白血症,即 Good's 综合征(GS)。尽管胸腺瘤和低丙种球蛋白血症的组合被认为足以诊断 Good's 综合征,但一些免疫缺陷临床表现明确的胸腺瘤患者的免疫球蛋白水平正常。我们描述了一例患者的病例,该患者患有 20 年胸腺瘤病史,经历了多次手术和化疗,反复感染,包括一次来自拟青霉属的罕见皮肤感染。该患者的丙种球蛋白水平一直正常。

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Is hypogammaglobulinemia a constant feature in Good's syndrome?低丙种球蛋白血症是否是 Good 综合征的一个固有特征?
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J Clin Immunol. 2014 Apr;34(3):283-8. doi: 10.1007/s10875-014-0014-7. Epub 2014 Mar 14.
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Good's syndrome and IgA monoclonal gammopathy of undetermined significance.古德综合征与意义未明的 IgA 单克隆丙种球蛋白病。
BMJ Case Rep. 2012 Nov 27;2012:bcr2012007601. doi: 10.1136/bcr-2012-007601.